Department of Dermatology, University of Pretoria, Republic of South
Africa.

Clin Exp Dermatol 1999 May;24(3):160-3 Abstract quote

Eighteen black South African patients with pityriasis rubra pilaris
were prospectively studied, nine adults and nine children; patients
with the classical types (namely I and III) constituted 75% of the group.
Follicular involvement was very prominent, especially in the children
with type III, but otherwise the clinical and histological findings
did not differ from those reported in white patients.

This is the first series of black African patients with this condition
to be reported.

DISEASE ASSOCIATIONS

CHARACTERIZATION

ARTHROPATHY

The relationship between pityriasis rubra pilaris and inflammatory
arthritis: case report and response of the arthritis to anti-tumor necrosis
factor immunotherapy.

Pityriasis rubra pilaris (PRP) refers to a group of erythematous, scaling
dermatologic conditions that have been associated with seronegative
arthritis.

We report a case of polyarthritis in a young man with PRP in which
magnetic resonance imaging suggested an entheseal-based pathology for
the joint disease. The arthritis, but not the skin condition, demonstrated
dramatic response to anti-tumor necrosis factor immunotherapy.

We report a unique case of atypical PRP beginning at 1 year of age
with associated severe arthropathy and osteoporosis. We further discuss
the clinical and histopathologic aspects of PRP, its possible etiology,
and other associated conditions.

A pityriasis rubra pilaris-like eruption has been described in patients
with dermatomyositis. These patients showed generalized follicular hyperkeratosis
and diffuse thickening of the palms and soles. Histopathological findings
consisted of keratotic plugging of the follicular infundibulum and features
of erector pili myositis.

We report on an 18-year-old woman with dermatomyositis. The diagnosis
was established by characteristic enzymatic alterations, electromyographic
pattern of myositis and the findings in a muscle biopsy, although the
patient had no evidence of muscular weakness during a follow-up of 14
years. She developed an erythematosus and squamous eruption associated
with diffuse palmoplantar keratoderma.

Histopathological features consisted of a papillomatous epidermis
with spicules of compact eosinophilic hyperkeratosis over the tips of
papillae that were not related to hair follicles. Pityriasis rubra pilaris-like
eruption seems to be a characteristic although uncommon cutaneous manifestation
in dermatomyositis.

An unusual presentation of dermatomyositis: the type Wong variant revisited.

We describe a 53-year-old white woman with dermatomyositis (DM) who
had additional clinical findings of pityriasis rubra pilaris (type Wong
dermatomyositis) with histopathologic features of both pityriasis rubra
pilaris (PRP) and porokeratosis.

Type Wong dermatomyositis was originally described in 11 patients
by Wong in 1969 and has been reported in 5 additional patients. This
is a rarely described phenomenon in which patients with DM develop cutaneous
hyperkeratotic lesions that resemble PRP and histologically show follicular
hyperkeratosis and hair follicle destruction. Arrector pilorum muscles
also show degenerative findings and myositis.

We believe that this is the first reported case of a patient with type
Wong DM who also has clinical and histologic features suggestive of
porokeratosis. This is important because of the association of adult-onset
dermatomyositis with internal malignancy and the well-documented association
of porokeratosis with immunosuppression. These clinical and histologic
findings serve as markers for malignancy in patients with DM. These
patients warrant a complete review of systems and investigation for
age-appropriate neoplasms as well as close long-term follow-up by dermatologists
to ensure that these cutaneous eruptions are not overlooked.

HEPATOCELLULAR CARCINOMA

Pityriasis rubra pilaris as an initial presentation of hepatocellular
carcinoma.

Sharma S, Weiss GR, Paulger B.

Division of Oncology, University of Texas Health Science Center
at San Antonio, 78284-7884, USA.

Dermatology 1997;194(2):166-7 Abstract quote

Pityriasis rubra pilaris (PRP) is a rare skin condition that is of
unknown etiology.

We present an unusual case of a patient with PRP as the sole initial
manifestation of hepatocellular cancer. The course of the patient's
illness and management of this case is discussed. Possible reasons for
this association are presented.

Recently, the occurrence of pityriasis rubra pilaris (PRP) has been
reported in patients with HIV infection. It presents different clinical
features, and has a poorer prognosis, than the classical adult type
of PRP.

We report the occurrence of severe PRP in an HIV-infected patient,
and review the previously reported cases of this association. We propose
the designation of a new category of PRP (type 6), characterized by
the presence of HIV infection, usually without immunosuppression, a
poor prognosis and response to treatment, and the development of nodulocystic
and lichen spinulosus lesions.

Pityriasis rubra pilaris is an uncommon hyperkeratotic, papulosquamous
disorder that has been reported in patients infected by HIV.

We recount a case of pityriasis rubra pilaris in an HIV-seropositive
man. A 36-year-old man with a history of ulcerative colitis and recurrent
otitis externa had diffuse psoriaform erythroderma. He was treated initially
with methotrexate and isoretinoin without clinical improvement. Skin
examination showed large, erythematous, orange, scaly patches on the
upper extremities and thickening of the nail beds. The palms and soles
were hyperkeratotic. Skin biopsy revealed changes that were consistent
with pityriasis rubra pilaris. Six months before the onset of symptoms,
results of an enzyme-linked immunosorbent assay (ELISA) and Western
Blot assay for HIV were negative. Six months after symptoms, results
of repeat enzyme-linked immunosorbent assay and Western blots for HIV
were positive (CD4+ T-cell count = 200 cells/ mm3). Clinical course
had been complicated by episodes of Staphylococcus aureus bacteremia,
mucocutaneous candidiasis, and development of localized squamous cell
carcinoma of the skin.

The increased severity of pityriasis rubra pilaris should prompt clinicians
to consider coinfection with HIV in patients who have disease that is
refractory to treatment. Clinicians also should remain vigilant for
the development of squamous cell carcinoma.

HIV-associated pityriasis rubra pilaris (PRP) or PRP type VI designates
a new distinctive entity reported in HIV patients. It is characterized
by cutaneous lesions of PRP and variable association with lesions of
acne conglobata, hidradenitis suppurativa and lichen spinulosus.

We report a patient with HIV-associated PRP which was treated by triple
antiretroviral therapy (zidovudine, lamivudin and saquinavir) with complete
response. The patient has remained free from symptoms for 20 months
of follow-up. We review the clinical features, pathology, evolution,
treatment and possible aetiology of this recently described entity.

At the time of initial diagnosis and treatment for upper limb MCC,
axillary nodal metastases were clinically evident. In the ensuing months,
she developed multiple rapidly progressing SCC and eventually a left
arm soft tissue deposit of metastatic MCC. Treatment involved multiple
courses of fractionated radiotherapy.

The salient clinical features and supporting evidence for this case
are presented.

PATHOGENESIS

CHARACTERIZATION

RETINOL BINDING PROTEIN

Pityriasis rubra pilaris and and retinol-binding protein.

Finzi AF, Altomare G, Bergamaschini L, Tucci A.

Br J Dermatol 1981 Mar;104(3):253-6 Abstract quote

Serum levels of retinol-binding protein (the specific carrier of vitamin
A) were measured in eleven patients with pityriasis rubra pilaris and
in some of their close relatives.

The level of retinol-binding protein was markedly reduced in every
patient, and in some of the relatives. It is postulated that defective
synthesis of retinol-binding protein is a biochemical marker for pityriasis
rubra pilaris, probably transmitted as a Mendelian dominant.

GROSS APPEARANCE/
CLINICAL VARIANTS

CHARACTERIZATION

GENERAL

Pityriasis rubra pilaris: a new proposed classification.

Piamphongsant T, Akaraphant R.

Institute of Dermatology, Bangkok, Thailand.

Clin Exp Dermatol 1994 Mar;19(2):134-8 Abstract quote

Clinical features of pityriasis rubra pilaris (PRP) were classified
into four types based on a study of 168 cases. All patients, except
one case of type IV, and 12 cases of type II, have salmon-coloured or
erythematous thick plaques on the palms and soles, which extended beyond
the dorsopalmar and plantar junctions.

Twenty-one children and 11 adults presenting with such plaques were
classified as type I PRP. Fifty-nine children and 27 adults presenting
with circumscribed scaly erythematous patches on the elbows and knees
were classified as type II. Twenty children and 16 adults with similar
patches involving large areas of the trunk which were not generalized
were classified as type III. Four children and 10 adults with exfoliative
erythroderma associated with diffuse follicular plugging were classified
as type IV. The diagnosis was confirmed by histopathology which showed
follicular plugging and focal parakeratosis. However, this was not seen
in type I.

This new clinical classification facilitates recognition of this type
of PRP despite the absence of follicular plugging. Two children, initially
classified as type I, showed gradual progression to type II on follow-up,
with two adults progressing to type III, and 1 adult to type IV. Similarly,
two adults classified as type II, progressed to type III, and 1 adult
into type IV. Associated findings were an adult of type II who developed
localized pustular psoriasis and another adult who developed ovarian
adenocarcinoma.

Four patients with pityriasis rubra pilaris are reported. The diagnosis
in each was based upon well-recognized clinical features. Two of them,
a mother and son, had the disease since childhood and were marked by
relative remission in spring and exacerbation in autumn. Moderate to
severe pruritus was a common dominator.

Erythroderma was a presenting feature in one case. Although histopathology
was considered imperative, it only supplemented the clinical expression.
Vitamin A in heavy dosage, supplemented by vitamin E and stanozolol
in tandem, was the mainstay of treatment.

We report a case of follicular keratosis with inflammatory changes,
consistent with a diagnosis of atypical juvenile pityriasis rubra pilaris.

An unusual feature was the occurrence of severe Staphylococcus aureus
folliculitis and furunculosis, a phenomenon rarely encountered in pityriasis
rubra pilaris and the other follicular keratoses. Standard antibiotic
and antiseptic treatment for chronic S. aureus infection was ineffective.

The patient was subsequently found to have hypogammaglobulinaemia,
and treatment with human polyvalent immunoglobulin infusions was successful
in eradicating the sepsis. It is therefore probable that the hypogammaglobulinaemia
played a pathogenic role in the development of cutaneous sepsis.

Juvenile acute pityriasis rubra pilaris (JAPRP) is a form of pityriasis
rubra pilaris (PRP) with particular clinical characteristics and course.
It is usually preceded by an infectious condition.

We report a boy, initially thought to have Kawasaki disease, and subsequently
diagnosed as having JAPRP, who made a satisfactory recovery. In this
case, prior antibiotic treatment made it impossible to show the presence
of any microorganism. However, the clinical characteristics of these
patients and the similarities with other disorders involving the skin
that are mediated by superantigens led us to think that these antigens
may be involved in the development of this disease. Also, in view of
the obvious differences between JAPRP and the other PRP, we suggest
that, in the future this disorder may be considered a separate entity
as a reactive exanthem.

A randomized clinical trial of 5% topical minoxidil versus 2% topical
minoxidil and placebo in the treatment of androgenetic alopecia in men.

BACKGROUND: Topical minoxidil solution 2% stimulates new hair growth
and helps stop the loss of hair in individuals with androgenetic alopecia
(AGA). Results can be variable, and historical experience suggests that
higher concentrations of topical minoxidil may enhance efficacy.

OBJECTIVE: The purpose of this 48-week, double-blind, placebo-controlled,
randomized, multicenter trial was to compare 5% topical minoxidil with
2% topical minoxidil and placebo in the treatment of men with AGA.

CONCLUSION: In men with AGA, 5% topical minoxidil was clearly superior
to 2% topical minoxidil and placebo in increasing hair regrowth, and
the magnitude of its effect was marked (45% more hair regrowth than
2% topical minoxidil at week 48). Men who used 5% topical minoxidil
also had an earlier response to treatment than those who used 2% topical
minoxidil. Psychosocial perceptions of hair loss in men with AGA were
also improved. Topical minoxidil (5% and 2%) was well tolerated by the
men in this trial without evidence of systemic effects.

FAMILIAL

Familial pityriasis rubra pilaris.

Vanderhooft SL, Francis JS, Holbrook KA, Dale BA, Fleckman P.

Department of Medicine, University of Washington School of Medicine,
Seattle, USA.

Arch Dermatol 1995 Apr;131(4):448-53 Abstract quote

BACKGROUND: Familial pityriasis rubra pilaris is a rare autosomal dominant
skin disorder. Four individuals from one family are described who demonstrate
clinical features compatible with a diagnosis of familial pityriasis
rubra pilaris. Results of light and electron microscopic, immunocytochemical,
and biochemical analysis of skin biopsy specimens from three of these
four individuals are presented.

OBSERVATIONS: All affected individuals demonstrated erythematous scaly
skin with follicular prominence and islands of sparing. Inheritance
was consistent with an autosomal dominant trait. Light and electron
microscopic findings were compatible with those reported in sporadic
cases of pityriasis rubra pilaris. Immunocytochemistry showed suprabasal
staining with monoclonal antibody AE1. Immunoblot analysis revealed
abnormal keratins with K6/16 expression, the possibility of an abnormal
K14 or K16, and a 45-kd acidic keratin not normally expressed in epidermis.
Because similar biochemical analyses have not been reported previously
in other cases of pityriasis rubra pilaris (familial or sporadic), comparisons
cannot be made.

CONCLUSIONS: The observations suggest that the cutaneous abnormality
in this family extends beyond clinical and morphological alterations
to abnormalities in biochemical markers of epidermal differentiation.

NAILS

The nails in adult type 1 pityriasis rubra pilaris. A comparison
with Sezary syndrome and psoriasis.

Sonnex TS, Dawber RP, Zachary CB, Millard PR, Griffiths AD.

J Am Acad Dermatol 1986 Nov;15(5 Pt 1):956-60 Abstract quote

The fingernails of twenty-four patients with classical adult (type
1) pityriasis rubra pilaris were examined for changes in nail morphology
and a comparison was made with the fingernail morphology of twenty-seven
consecutive psoriatic patients with nail changes.

Distal yellow-brown discoloration, subungual hyperkeratosis, nail plate
thickening, and splinter hemorrhages indicate a diagnosis of type 1
pityriasis rubra pilaris rather than psoriasis, while onycholysis (particularly
marginal), salmon patches, small pits, and larger indentations of the
nail plate indicate a diagnosis of psoriasis. Histology demonstrated
that a nail biopsy would be a useful diagnostic procedure if nails were
involved in isolation but provided no additional diagnostic features
to those found from biopsy specimens of involved skin in the two conditions.

The similarity in nail morphology between the type 1 pityriasis rubra
pilaris patients and five patients with chronic erythroderma resulting
from Sezary syndrome indicated that these changes may represent a nonspecific
reaction pattern that may result from prolonged erythema of the proximal
nail bed and matrix. The rough nails (trachyonychia) described in advanced
Sezary syndrome were not observed in any of our patients.

HISTOLOGICAL TYPES

CHARACTERIZATION

GENERAL

The clinical and histomorphological features of pityriasis rubra pilaris.
A comparative analysis with psoriasis.

Pityriasis rubra pilaris (PRP) is an idiopathic erythematous scaling
eruption which can be difficult to distinguish from psoriasis.

We explored the clinical features, including background medical illnesses
and potential triggers in patients with PRP, and also its histomorphologic
spectrum. Patients with PRP were selected by natural language search
of dermatopathology databases containing 250,000 cases accessioned over
a 3-year period. A detailed medical history was obtained on each patient
via office chart review and patient interviews.

We compared the histologic findings in skin biopsies from patients
with PRP to those of biopsies matched for site and age from control
patients with psoriasis. Certain aspects of the clinical presentations
encountered in the study group patients have not been previously emphasized,
including eruptions compatible with a vesiculobullous disorder in 4
patients. Several patients had evidence of underlying iatrogenic or
systemic immune dysregulatory states and certain triggers could be isolated
such as emotional stress and prior exposure to microbial pathogens.

Histomorphologic features predictive of PRP included follicular plugging,
an increased granular cell layer, and acantholysis. While seen in a
minority of biopsies of PRP, morphologic discriminators supportive of
a diagnosis of psoriasis included neutrophils in the parakeratotic scale
crust, and dermal papillae capillary ectasia with vessels lying in direct
apposition to the epidermis. Other morphologic features were encountered
in biopsies of PRP which have received little emphasis in the dermatopathology
literature, including epithelial atrophy, significant dermal inflammation,
the presence of eosinophils and plasma cells within the inflammatory
infiltrates, an abnormal granular cell layer, and epidermolytic hyperkeratosis,
albeit none were statistically signficant predictors of PRP over psoriasis.
Nevertheless, their identification emphasizes the varied histologic
spectrum of PRP. The almost ubiquitous finding of acantholysis and the
presence of an abnormal granular cell layer in many biopsies of PRP
suggests that abnormal keratins or defects in the retinol signalling
pathway may be of pathogenetic importance.

VARIANTS

ACANTHOLYSIS

PRP with subcorneal acantholysis: Case report and review.

Sebastian A, Koff AB, Goldberg LJ.

Department of Pathology, Drexel College of Medicine, Philadelphia, PA.

J Cutan Pathol. 2009 Jul 7. Abstract quote

Although the clinical findings of pityriasis rubra pilaris (PRP) are striking, the microscopic diagnosis can be difficult because changes can be subtle and can overlap with other skin diseases. Suprabasal acantholysis can be seen in PRP, mimicking pemphigus vulgaris.

Department of Dermatology, University of Pennsylvania, Philadelphia,
USA.

J Cutan Pathol 1996 Jun;23(3):270-4 Abstract quote

Two patients developed a papulosquamous eruption in a widespread distribution
which progressed with islands of sparing of uninvolved skin characteristic
clinically of adult-onset pityriasis rubra pilaris (PRP).

Biopsies from both patients showed multiple areas of nonfollicular
and follicular suprabasilar and intra-epidermal acantholysis with minimal
dyskeratosis. They also showed the usual histologic features of PRP
with a thickened orthokeratosis and parakeratosis, a retained and sometimes
thickened granular cell layer, and psoriasiform epidermal hyperplasia
with a perivascular lymphohistiocytic infiltrate in the superficial
dermis.

Two previous patients with PRP have been reported with nonfollicular,
focal acantholytic dyskeratosis and both were interpreted as most likely
representing an incidental finding. We believe the acantholysis in these
two cases is related to the disease process, and in our second patient,
was helpful in establishing the diagnosis.

Pityriasis rubra pilaris with acantholysis and lichenoid histology.

Hashimoto K, Fedoronko L.

Department of Dermatology & Syphilology, Wayne State University
School of Medicine, Detroit, Michigan, USA.

Am J Dermatopathol 1999 Oct;21(5):491-3 Abstract quote

Acantholytic foci have been reported several times in pityriasis rubra
pilaris (PRP). Lichenoid tissue reactions were also mentioned in the
literature regarding PRP. We report a 58-year-old patient who, after
having colon cancer, had PRP with biopsies showing acantholytic lesions
and a heavy lichenoid lymphocytic infiltration.

Investigation by serial sectioning of the acantholytic lesion suggested
an involvement of the intraepidermal eccrine duct and further investigation
with carcinoembryonic antigen (CEA) staining demonstrated a CEA-positive
eccrine duct in the acantholytic foci.

We suggest that acantholysis in PRP is induced by proteolytic enzymes,
urea, and other substances in eccrine sweat in keratin-plugged acrosyringia.
This patient had a combination of three relatively rare features of
PRP-acantholysis, lichenoid reaction, and a cancer background.

Focal acantholytic dyskeratosis (FAD) is a distinctive histologic pattern
characterized by suprabasilar clefts surrounding dermal papillae (villi),
acantholytic and dyskeratotic cells at all levels of the epidermis,
hyperkeratosis, and parakeratosis.

The features of FAD are typically seen in Darier's disease, warty dyskeratoma,
and transient acantholytic dermatosis; they are also present in a variety
of cutaneous neoplastic and nonneoplastic lesions. FAD, however, has
not been previously described in lesions of inflammatory dermatoses.

We report a case of FAD occurring in lesions of pityriasis rubra pilaris
(PRP). To the best of our knowledge, this is the first reported case
of this kind. We also review the pertinent literature.

SPECIAL STAINS/IMMUNOPEROXIDASE/
OTHER

CHARACTERIZATION

SPECIAL STAINS

Pityriasis rubra pilaris: a clinico-pathological and therapeutic study
with special reference to histochemistry, autoradiography, and electron
microscopy.

Braun-Falco O, Ryckmanns F, Schmoeckel C, Landthaler M.

Arch Dermatol Res 1983;275(5):287-95 Abstract quote

Five patients with pityriasis rubra pilaris (PRP) were analyzed by
means of light and electron microscopy as well as by histochemistry
and autoradiography. The results were compared with findings in psoriasis
vulgaris.

In PRP we found a moderate increase of the labeling index of epidermal
cells, a highly increased labeling index of dermal infiltrating cells,
and a mild spongiosis, and in the stratum granulosum, a decreased number
of tonofilaments and an increased number of keratinosomes. The horny
layer in PRP showed a pronounced histochemical and electron microscopical
parakeratosis, even when histological parakeratosis was absent. In contrast
with psoriasis vulgaris, there was no exocytosis of polymorphonuclear
leucocytes into the epidermis, the papillomatosis index was normal,
and there were no tortuous capillaries in the dermal papillae.

The stratum granulosum was always present and sometimes thickened,
showing electron microscopical changes different from those referred
to in psoriasis. These changes point to a relatively distinct pattern
of epidermal changes in PRP.

We present a case of pityriasis rubra pilaris (PRP)-like eruption,
in association with human immunodeficiency virus (HIV) infection, clinically
characterized by follicular papules with some elongated spines and comedo-like
lesions. The lesions were located on the back, flanks, and proximal
extremities. Orthokeratotic follicular plugs and perifollicular mucinous
degeneration were consistently observed on three biopsies. The most
characteristic histological features of PRP were absent.

To our knowledge, this is the first reported case of this kind. Such
a case could suggest an atypical PRP modified by HIV infection. It is
also possible that it represents a new entity associated with advanced
HIV infection (horny perifollicular mucinosis).

BACKGROUND: There are conflicting opinions about the diagnostic value
of skin biopsy in erythrodermic psoriasis.

OBJECTIVE: The purpose of the present study was to establish the specificity
of the histopathologic changes of psoriatic erythroderma.

METHODS: We reviewed 52 skin biopsies from 45 erythrodermic patients
having a final diagnosis of psoriasis on the basis of combined clinical
and laboratory data, in addition to response to therapy and follow-up.
In 5 patients, erythroderma was the presenting sign of psoriasis. A
control group of nonpsoriatic erythrodermic patients was also included
in the study.

RESULTS: Among the group of patients with a discharge diagnosis of
psoriatic erythroderma, the histopathologic changes were specific for
psoriasis in 40 cases (88%). The changes of early macular and squamous
lesions of psoriasis were more often found in the biopsy specimens of
our series than those of fully developed or late lesions of psoriasis.
They included mainly slight epidermal hyperplasia, focal disappearance
of the granular layer, mounds of parakeratosis and extravasated erythrocytes
within edematous dermal papillae associated with perivascular and interstitial
infiltration of lymphocytes and histiocytes.

CONCLUSION: When features of early lesions of psoriasis are found during
the evaluation of a biopsy specimen from a patient with a clinically
nonspecific erythroderma, the dermatopathologist should be aware that
this patient could have psoriasis and a renewed anamnesis and a close
follow-up should be made.

PROGNOSIS AND TREATMENT

CHARACTERIZATION

PROGNOSTIC FACTORS

GENERAL

Pityriasis rubra pilaris in childhood: a long-term study of 29 cases.

Gelmetti C, Schiuma AA, Cerri D, Gianotti F.

Pediatr Dermatol 1986 Dec;3(6):446-51 Abstract quote

Pityriasis rubra pilaris (PRP) is a dermatosis of unknown origin
with a limited frequency in childhood (0.2% of our hospitalized pediatric
patients).

During the last 20 years we have observed 31 new cases; follow-up
has been conducted in 29 children. The acute self-resolving form seems
to be the most frequent in children, compared to adults. No cases of
hereditary PRP have been observed, and no relationship between the severity
of PRP and its prognosis has been reported.

Since juvenile PRP has a relatively rapid course and a spontaneous
resolution (a few months), it seems unnecessary to use potentially harmful
drugs. Synthetic retinoids may be employed in patients whose disease
is both persistent and disabling.

BACKGROUND: Pityriasis rubra pilaris (PRP) often has a devastating
impact on the lives of patients. Descriptions of its histopathologic
features are not uniform. Finding a successful therapy can be challenging.

OBJECTIVE: Our purpose was to examine the histopathologic features
and response of patients to our standard therapy of an oral retinoid
and concomitant or later addition of low-dose weekly methotrexate.

METHODS: A retrospective chart review was done on 24 patients with
PRP seen from March 1986 to March 1996. Biopsy specimens from 19 patients
were reexamined. Telephone follow-up was conducted to determine maintenance
of remission.

RESULTS: All patients had the adult acquired form of PRP. Biopsy
specimens from nine patients were characterized by prototypical findings
of PRP, while the others included both typical and other features. Twenty-two
patients were treated with either isotretinoin, 40 mg twice daily, or
etretinate, 25 to 75 mg/day. Six patients with more disabling involvement
had low-dose weekly methotrexate ranging from 5 to 30 mg started concurrently.
Five patients had weekly methotrexate added at a later time. Seventeen
patients showed 25% to 75% response after 16 weeks of therapy. All patients
whose skin cleared maintained their remission.

CONCLUSION: Initial oral retinoid plus concurrent or later low-dose
weekly methotrexate resulted in 25% to 75% improvement of PRP in 17
of 24 patients after 16 weeks of therapy. Some of the atypical features
seen in biopsy specimens emphasize the importance of clinical and histopathologic
correlation in establishing the diagnosis.

TREATMENT

GENERAL

Treatment of classic pityriasis rubra pilaris.

Dicken CH.

Department of Dermatology, Mayo Clinic, Rochester, MN 55905.

J Am Acad Dermatol 1994 Dec;31(6):997-9 Abstract quote

BACKGROUND: Treatment of classic pityriasis rubra pilaris, which
almost always progresses to a generalized erythroderma with marked,
often disabling keratoderma of the palms and soles, remains problematic.

OBJECTIVE: Our purpose was to evaluate the results of treatment
in a recent period during which the retinoid group of drugs has been
available.

METHODS: The clinical course of 75 patients with classic pityriasis
rubra pilaris seen from 1982 to 1992 was reviewed.

RESULTS: Of 15 patients treated with isotretinoin, 10 had complete
and 2 had partial clearing. Of six treated with etretinate, four had
clearing. All eight patients treated with methotrexate had a favorable
response. Other forms of treatment, including Goeckerman regimen, corticosteroids,
vitamin A, and cyclosporine, were ineffective.

CONCLUSION: Early diagnosis and early treatment with retinoids appear
to offer the best chance for clearing of pityriasis rubra pilaris. If
retinoids fail or cannot be used, methotrexate should be considered.

OBJECTIVE: We attempted to determine the efficacy of CsA in the
treatment of classic adult-type PRP.

METHODS: Three patients with classic adult-type PRP were treated
with 5 mg/kg/day CsA.

RESULTS: A sustained clinical response was achieved within 2-4 weeks
of therapy. Relapses were noted when the CsA dose was decreased to 1.2
mg/kg/day.

CONCLUSION: CsA should be considered in the treatment of classical
adult-type PRP

ISOTRETINOIN

Isotretinoin treatment of pityriasis rubra pilaris.

Dicken CH.

J Am Acad Dermatol 1987 Feb;16(2 Pt 1):297-301 Abstract
quote

Five patients with pityriasis rubra pilaris were treated with isotretinoin
from September 1982 through 1985. Isotretinoin at an average dose of
1.16 mg/kg/day for 16 to 24 weeks caused complete or almost complete
clearing in four of the five patients.